CLASSIFICATION, CLINICAL PICTURE, AND DIFFERENT APPROACHES TO SURGICAL TREATMENT IN CRANIOPHARYNGIOMAS (LITERATURE REVIEW)

Аннотация

40 academic sources reflecting the classification, diagnostics, clinics and treatment methods of craniopharyngiomas (CP) were analayzed. Craniopharyngiomas (CP) are rare, slow-growing, epithelial tumors that develop from remnants of Rathke’s pouch cells. Primarily located in the sellar and parasellar regions and close to vital neurovascular structures (optic chiasm, hypothalamus, pituitary thus making complete surgical resection challenging. Despite being histologically benign, these tumors tend to infiltrate critical parasellar structures and can behave aggressively, leading to serious disability and even mortality (even after successful treatment) [6,24,29]. Histologically, craniopharyngiomas are classified into adamantinomatous (ACP) and papillary (PCP) types, found in children and adults, respectively. While the genetic causes of craniopharyngiomas are not fully understood, ACP development is associated with CTNNB1 mutations, which result in β- catenin forms resistant to degradation and activation of the WNT/β-catenin pathway. PCP, on the other hand, is often linked to the BRAF V600E mutation[6,24].Currently, complete tumor resection provides the best outcomes in CP treatment, and the most optimal surgical approach is selected based on the tumor’s location, size, consistency, and the patient’s overall condition. This article reviews surgical approaches to treating craniopharyngiomas andattempts to identifythe mosteffective approach. Keywords. Craniopharyngioma, Rathke’s pouch remnants, adamantinomatous, papillary, preinfundibular, transinfundibular, retroinfundibular, cyst aspiration, extended endoscopic transnasal transsphenoidal approach. Epidemiologyof Craniopharyngiomas Craniopharyngiomas, or Rathke's pouch tumors, are considered a congenital condition. They develop from embryonic cells—protrusions of the mucous membrane of the posterior 98,7:ROTCAFTCAPMI .SECNEICSLACIDEM ,5202, enuJ,5emuloV gro.srehsilbupcimedaca.www pharyngeal wall, known as Rathke's pouch. In 1925, Cushing described the clinical picture of these tumors and gave them their name. Craniopharyngiomas are relatively rare among brain tumors [2]. The incidence is 0.13 cases per 100,000 people annually. They account for approximately 2- 5% of all primary brain tumors and 5.6-15% of brain tumors in children [4,7,11]. Despite craniopharyngiomas being the most common tumors affecting the hypothalamic-pituitary system in children, nearly half of all cases are diagnosed in adults. These tumors can occur at any age, including prenatal and neonatal periods, and have a bimodal age distribution: the highest incidence is observed in children aged 5-14 years andadults aged 50-74 years [4]. Pathogenesis ofCraniopharyngiomas The pathogenesis of craniopharyngiomas is not fully understood. According to one source, these tumors develop as a result of neoplastic transformation of embryonal squamous cells in the craniopharyngeal canal (adamantinomatous type) [32], while another source suggests they result from the metaplasia of adenohypophyseal cells in the pituitary stalk or gland (papillary type) [17]. Some of these tumors have a monoclonal origin and exhibit various chromosomal abnormalities, including translocations, deletions, and increased DNA copy number [6,9,29]. Theexact causes that triggerthe blastomatousprocess are stillunknown. Craniopharyngiomas can develop in any area of pituitary formation, but they most commonlyarise from thefollowingsources: 1. Epithelial cell clusters at the floor of the third ventricle and the pituitary stalk— thesetumors grow in thesuprasellar area. 2. Cell clusters in the pituitary stalk (under the diaphragm)—such craniopharyngiomas may be located within the Turkish saddle (intrasellar), extend beyond it, orcombineboth variants (intrasuprasellar). 3. Epithelial cell clusters in the sphenoid sinus—these tumors are called intrasphenoidal [2]. Classificationof Craniopharyngiomas 98,7:ROTCAFTCAPMI .SECNEICSLACIDEM ,5202, enuJ,5emuloV gro.srehsilbupcimedaca.www According to the World Health Organization (WHO) classification, there are two morphological types of craniopharyngiomas: adamantinomatous and papillary. Both types are classified as Grade I tumors in terms of malignancy. In children, 95% of craniopharyngiomas are of the adamantinomatous type, whereas in adults, 35% of tumors areof thepapillary (squamous)type (Karavitaki, Merchant, 2005). Several classifications ofcraniopharyngiomas exist,depending ontheirlocalization. For pediatric craniopharyngiomas, a special MRI classification system has been proposed, where themain criterion isthedegree ofinvolvement ofhypothalamic structures. M.G. Yasargil classified craniopharyngiomas based on their spread: Intrasellar, Infradiaphragmatic, Intra-suprasellar, Infra-supradiaphragmatic, Suprasellar, Parachiasmal, Extraventricular, Intra- and extraventricular, Paraventricular (relative to the third ventricle), andpurely intraventricular (intraventricular) craniopharyngiomas [33].